Pauci-immune crescentic glomerulonephritis in limited cutaneous systemic sclerosis.

Abstract:

:Pauci-immune necrotizing glomerulonephritis is a form of a rapidly progressive glomerulonephritis that is associated with the anti-neutrophil cytoplasmic antibodies (ANCA). ANCA-associated pauci-immune glomerulonephritis may be secondary to rheumatic diseases. We report on a patient with limited cutaneous systemic sclerosis who presented with subacute onset of nephritic syndrome and renal insufficiency. Her myeloperioxidase ANCA titer was elevated and renal biopsy confirmed pauci-immune crescentic glomerulonephritis, which was stabilized with high-dose glucocorticoid and cyclophosphamide. ANCA-associated necrotizing glomerulonephritis is rarely reported in systemic sclerosis and a systematic review is presented.

journal_name

Clin Rheumatol

journal_title

Clinical rheumatology

authors

Chan PT,Mok CC

doi

10.1007/s10067-012-2010-8

subject

Has Abstract

pub_date

2012-08-01 00:00:00

pages

1273-7

issue

8

eissn

0770-3198

issn

1434-9949

journal_volume

31

pub_type

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